Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes

Background: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular...

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Main Authors: Antonella Diamanti, Giacomo Calvitti, Diego Martinelli, Emma Santariga, Teresa Capriati, Giulia Bolasco, Lorenzo Iughetti, Arturo Pujia, Daniela Knafelz, Giuseppe Maggiore
Format: Article
Language:English
Published: MDPI AG 2021-02-01
Series:Nutrients
Subjects:
Online Access:https://www.mdpi.com/2072-6643/13/3/786
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spelling doaj-485528230c0c4f7fbaf0c5b29422e4da2021-02-28T00:03:36ZengMDPI AGNutrients2072-66432021-02-011378678610.3390/nu13030786Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive CausesAntonella Diamanti0Giacomo Calvitti1Diego Martinelli2Emma Santariga3Teresa Capriati4Giulia Bolasco5Lorenzo Iughetti6Arturo Pujia7Daniela Knafelz8Giuseppe Maggiore9Hepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, ItalyPediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, 41121 Modena, ItalyMetabolic Diseases Unit, “Bambino Gesù” Children Hospital, 00165 Rome, ItalyClinical Nutrition Unit, Department of Medical and Surgical Sciences, University Magna Graecia, 88100 Catanzaro, ItalyHepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, ItalyHepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, ItalyPediatric Unit, Department of Medical and Surgical Sciences for Mothers, Children and Adults, University of Modena and Reggio Emilia, 41121 Modena, ItalyClinical Nutrition Unit, Department of Medical and Surgical Sciences, University Magna Graecia, 88100 Catanzaro, ItalyHepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, ItalyHepatology Gastroenterology and Nutrition Unit, “Bambino Gesù” Children Hospital, 00165 Rome, ItalyBackground: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops. Methods: We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: “intestinal failure” OR “home parenteral nutrition” OR “short bowel syndrome” OR “chronic pseudo-obstruction” OR “chronic intestinal pseudo-obstruction” OR “autoimmune enteropathy” OR “long-term parenteral nutrition”. Results: We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion. Conclusions: The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management.https://www.mdpi.com/2072-6643/13/3/786intestinal failureenteral nutritionparenteral nutrition
collection DOAJ
language English
format Article
sources DOAJ
author Antonella Diamanti
Giacomo Calvitti
Diego Martinelli
Emma Santariga
Teresa Capriati
Giulia Bolasco
Lorenzo Iughetti
Arturo Pujia
Daniela Knafelz
Giuseppe Maggiore
spellingShingle Antonella Diamanti
Giacomo Calvitti
Diego Martinelli
Emma Santariga
Teresa Capriati
Giulia Bolasco
Lorenzo Iughetti
Arturo Pujia
Daniela Knafelz
Giuseppe Maggiore
Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes
Nutrients
intestinal failure
enteral nutrition
parenteral nutrition
author_facet Antonella Diamanti
Giacomo Calvitti
Diego Martinelli
Emma Santariga
Teresa Capriati
Giulia Bolasco
Lorenzo Iughetti
Arturo Pujia
Daniela Knafelz
Giuseppe Maggiore
author_sort Antonella Diamanti
title Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes
title_short Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes
title_full Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes
title_fullStr Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes
title_full_unstemmed Etiology and Management of Pediatric Intestinal Failure: Focus on the Non-Digestive Causes
title_sort etiology and management of pediatric intestinal failure: focus on the non-digestive causes
publisher MDPI AG
series Nutrients
issn 2072-6643
publishDate 2021-02-01
description Background: Intestinal failure (IF) is defined as reduction in functioning gut mass below the minimal amount necessary for adequate digestion and absorption. In most cases, IF results from intrinsic diseases of the gastrointestinal tract (digestive IF) (DIF); few cases arise from digestive vascular components, gut annexed (liver and pancreas) and extra-digestive organs or from systemic diseases (non-digestive IF) (NDIF). The present review revised etiology and treatments of DIF and NDIF, with special focus on the pathophysiological mechanisms, whereby NDIF develops. Methods: We performed a comprehensive search of published literature from January 2010 to the present by selecting the following search strings: “intestinal failure” OR “home parenteral nutrition” OR “short bowel syndrome” OR “chronic pseudo-obstruction” OR “chronic intestinal pseudo-obstruction” OR “autoimmune enteropathy” OR “long-term parenteral nutrition”. Results: We collected overall 1656 patients with well-documented etiology of IF: 1419 with DIF (86%) and 237 with NDIF (14%), 55% males and 45% females. Among DIF cases, 66% had SBS and among NDIF cases 90% had malabsorption/maldigestion. Conclusions: The improved availability of diagnostic and therapeutic tools has increased prevalence and life expectancy of rare and severe diseases responsible for IF. The present review greatly expands the spectrum of knowledge on the pathophysiological mechanisms through which the diseases not strictly affecting the intestine can cause IF. In view of the rarity of the majority of pediatric IF diseases, the development of IF Registries is strongly required; in fact, through information flow within the network, the Registries could improve IF knowledge and management.
topic intestinal failure
enteral nutrition
parenteral nutrition
url https://www.mdpi.com/2072-6643/13/3/786
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